GASTROINTESTINAL ANGIODYSPLASIA IN CONGENITAL PLATELET DYSFUNCTION

We herein report three cases of repeated massive bleeding from the sto mach and small bowel. One patient suffered from both thrombasthenia (t ype II) and von Willebrand disease (type 1) simultaneously. Two others had Bernard Soulier's syndrome (BSS). One patient with BSS had bleedi ng from gastric angiodysplasia and was treated endoscopically by clipp ing. The other patients had massive bleeding from the small intestine, and had partial resection of the affected small intestine. Histologic ally, irregular dilatation and proliferation of the blood vessels were demonstrated in the submucosa in bleeding spots from a resected small intestine, and these findings were consistent with the features of ac quired angiodysplasia. The development of gastrointestinal angiodyspla sia may not only be associated with a dysfunction of von Willebrand fa ctor but also with that of platelets.